Goodpasture's syndrome

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  • Pulmonary-renal syndrome


  1. Goodpasture's syndrome
  2. ANCA small-vessel vasculitis
  3. Wegener's granulomatosis
  4. Microscopic polyangitis
  5. Churg-Strauss syndrome
  6. Henoch-Schönlein purpura
  7. Cryoglobulinemia
  • D.D.
    • Lupus GN plus pneumonitis or pneumonia
    • Uremia plus pneumonia


  • Diagnostic triad
    • RPGN + Lung hemorrhage (hemoptysis) + anti-GBM antibody
    • Diagnosis
      • Serum anti-GBM antibody detected by antibody to α3 ~NC1 domain of type IV collagen
        • Serum anti-GBM antibody may be positive in patients with paraneoplastic syndrome if nonspecific anti-GBM antibodies are used
      • 10-15% p'ts also have serum ANCA to myeloperoxidase
        • Good prognosis
    • It appears in two age groups: in young men in late 20s and in men and women in 60–70s
    • Disease in the younger age group is usually explosive, with hemoptysis, a sudden fall in hemoglobin, fever, dyspnea, and hematuria. Hemoptysis is largely confined to smokers
    • Treatment
      • Plasmapheresis (for lung hemorrhage), steroids, cyclophosphamide
      • Renal transplantation (wait for 6 months and until serum antibodies are undetectable)
    • Prognosis
      • Poor if >50% crescents, advanced fibrosis, serum creatinine >5–6 mg/dL, oliguria, need for acute dialysis